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Understanding the Fontan Operation�&�Canadian Fontan Connection��

Dr. Jasmine Grewal MD, FRCPC

Professor, University of British Columbia

Director, Virani Provincial Adult Congenital Heart Program

Vancouver, B.C.

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“Normal” Heart

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Univentricular Heart

V

RA

LA

Tricuspid Atresia
Hypoplastic left heart syndrome
Unbalanced atrio-ventricular septal defect
Double outlet right ventricle
Double inlet left ventricle
Pulmonary atresia

RA

V

LA

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Univentricular Heart

1 in every 3000 children born in North America

Similar prevalence to CYSTIC FIBROSIS (1/3600)

Very poor prognosis with over 70% of individuals dying by 16 years of age

(if unrepaired)

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The Problem?

SVC

IVC

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Surgical Progress

80% now undergo a Fontan. It involves diverting the venous blood from the right atrium to the pulmonary arterieswithout passing through the morphologic right ventricle. It was initially described in 1971 by Dr. Francois Marie Fontan (1929 – ) from Bordeaux, France and Dr Guillermo Kreutzer from Buenos Aires, Argentina(1934 – ) separately as a surgical treatment for tricuspid atresia.^[1] ^[2] The Fontan procedure has more recently been used in pediatric situations where an infant only has a single effective ventricle, either due to lack of a heart valve (e.g. tricuspid ormitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex congenital heart disease where a bi-ventricular repair is impossible or inadvisable. Fontan was the first to completely bypass the right heart in a human subject and to channel both the IVC and SVC blood to the pulmonary arteries. He unsuccessfully attempted to bypass the right heart in healthy dogs. However, he reasoned that it may work in patients with tricuspid atresia because the right atrium is thicker and more muscular in humans than in canines and hence better able to perform a contractile function. He also believed that valved conduits were essential to prevent the blood from going in the opposite direction (ie, into the IVC). Therefore, he incorporated one valved homograft between the IVC and the right atrium and another valved homograft between the right atrium and the left pulmonary artery.

The final operation consisted of the classic Glenn SVC–to–right pulmonary artery operation as well as the right atrium–to–left pulmonary artery connection with the 2 homografts, as described. He published his results from the first three patients in 1971.^[2]

Kreutzer et al (1973) offered a modification almost immediately.^[4]They described a connection between the right atrial appendage and the main pulmonary artery accomplished by means of a pulmonary homograft or the patient's own pulmonary annulus but without a valved homograft at the IVC–right atrial junction. In this situation, the continuity of the pulmonary arteries is maintained.

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Fontan Operation – Early days

Atrio-pulmonary Fontan

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Fontan Operation

Bidirectional Glenn

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Fontan Completion

Lateral Tunnel Fontan

Extra-Cardiac Fontan

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Fontan Fenestration

Lateral Tunnel Fontan

Extra-Cardiac Fontan

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Mission

To create a comprehensive lifespan registry of Fontan patients living in Canada

To support Canadian and International research collaborations that improve the lives of Fontan patients

Vision

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CANFON�Canadian Fontan Connection

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CANFON – Central Coordination

St. Paul’s Hospital, Vancouver, B.C.

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CANFON – Data house

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Recruitment/Review

CANFON Advisory Committee

(Patient and Parent Advocacy)

CANFON Registry

Steering Committee

CANFON

CARE PROVIDERS/RESEARCHERS

PATIENTS/FAMILIES

Peer to Peer support

Annual Reports

International meetings

CACH-Net

CCS

CCPCRN/CCI

Fontan Website

Fontan Annual Report

Fontan Education Days

CCHA

CPCA

Patient reported outcomes/QOL

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Thank you for your time!